S-b-aminoisobutyric acid

From NuGOwiki

(Redirected from HMDB02166)

The NuGOwiki Metabolite Database is a joint initiative of NuGO and HMDB

Taxonomy Navigation Box; search by

S-b-aminoisobutyric acid
2D structure for S-b-aminoisobutyric acid
Chemical Name
Chemical Formula	C₄H₉NO₂
CAS Number	4249-19-8
Chemical Information	HMDB02166
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Urine
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 1.03 +/- 0.34 uM Urine: 10.0 +/- 9.0 umol/mmol creatinine Urine: 4.3 +/- 2.9 umol/mmol creatinine Urine: 7.0 +/- 6.6 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

Authors:
Affiliations:

Introduction

guidelines
Beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-Aminoisobutyric acid is normally low in urine as beta-Aminoisobutyric acid is further catabolized by b-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-Aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-Aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-Aminoisobutyric acid have been observed in individuals with a deficiency of R (-) -b-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-Aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation and in a number of malignancies. The S-enantiomer of beta-Aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that an altered homoeostasis of b-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to b-alanine and the R-enantiomer of beta-Aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-Aminoisobutyric acid and the subsequent accumulation of S-beta-Aminoisobutyric acid in plasma. (PMID: 14705962, 14292857, 14453202)